Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis II) fibroblasts.
about
Sialidosis: a review of human neuraminidase deficiency.Electrophoretic abnormalities of lysosomal enzymes in mucolipidosis fibroblast linesTwo clonal cell populations (mosaicism) in a 46,XY male with mucolipidosis II (I-cell disease)--an autosomal recessive disorder.Mutant defective in processing of an enzyme located in the lysosome-like vacuole of Saccharomyces cerevisiae.Chick embryo fibroblasts produce two forms of hyaluronidase.Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man.Isolation and characterization of phosphorylated oligosaccharides from alpha-N-acetylglucosaminidase that are recognized by cell-surface receptors.Quantitative Proteome Analysis of Mouse Liver Lysosomes Provides Evidence for Mannose 6-phosphate-independent Targeting Mechanisms of Acid Hydrolases in Mucolipidosis II.Cherry-red spot myoclonus syndrome and alpha-neuraminidase deficiency: neurophysiological, pharmacological and biochemical study in an adultUrinary lysosomal hydrolases in mucolipidosis II and mucolipidosis IIIProperties of acid beta-D-galactosidase isolated from I-cell disease brain and spleen.Neurochemical abnormality in I-cell disease: chemical analysis and a possible importance of beta-galactosidase deficiency.Correction of I-cell defect by hybridization with lysosomal enzyme deficient human fibroblasts.I-cell disease. A hypothesis for the structure of the carbohydrate recognition site on beta-D-N-acetylhexosaminidase.Cellular transport of lysosomal enzymes: an alternative hypothesis.Demonstration of altered acidic hydrolases in fibroblasts from patients with mucolipidosis II by lectin titration.Cellular localization of neuraminidases in cultured human fibroblasts.Structure of nine sialyl-oligosaccharides accumulated in urine of eleven patients with three different types of sialidosis. Mucolipidosis II and two new types of mucolipidosis.Oligosaccharide and ganglioside neuraminidase activities of mucolipidosis I (sialidosis) and mucolipidosis II (I-cell disease) fibroblasts.
P2860
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P2860
Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis II) fibroblasts.
description
1976 nî lūn-bûn
@nan
1976年の論文
@ja
1976年論文
@yue
1976年論文
@zh-hant
1976年論文
@zh-hk
1976年論文
@zh-mo
1976年論文
@zh-tw
1976年论文
@wuu
1976年论文
@zh
1976年论文
@zh-cn
name
Increased levels of sialic aci ...... mucolipidosis II) fibroblasts.
@en
Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease
@nl
type
label
Increased levels of sialic aci ...... mucolipidosis II) fibroblasts.
@en
Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease
@nl
prefLabel
Increased levels of sialic aci ...... mucolipidosis II) fibroblasts.
@en
Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease
@nl
P2093
P1476
Increased levels of sialic aci ...... mucolipidosis II) fibroblasts.
@en
P2093
P304
P356
10.1016/0006-291X(76)90267-9
P407
P577
1976-07-01T00:00:00Z